I'm Aware That I'm Rare: Roberta Keller, MD


Roberta Keller, MD discusses the importance of multidisciplinary comprehensive care centers for children with chronic cardiopulmonary conditions. Dr. Keller is a neonatologist from UCSF’s Benioff Children’s Hospital in San Francisco.

Transcript:

I’m Roberta Keller. I’m a professor of pediatrics at the University of California San Francisco Benioff Children’s Hospital in San Francisco. I’m a neonatologist, and I specialize in evaluation and management of newborns and children with cardiopulmonary disorders.

I think as my practice has continued over the years I’ve become more and more interested in the longer term care of children who survive cardiopulmonary disorders in the newborn period. I think if we look at the model of other children who have complex medical disorders such as spina bifida we see that really multidisciplinary care and multidisciplinary clinics are the standard for providing support and medical care for families and children with spina bifida. As I’ve gone on in my practice and in my career, I’ve come to realize how important that really is for children with other disorders.

One example is children who survive with congenital diaphragmatic hernia, which is a birth defect that affects the development of the diaphragm on one side of the chest, but also as a result the development of the lung and the vascular bed on both sides of the chest throughout the lung. So what we see with children who survive that condition is that they often have chronic cardiopulmonary conditions which are even more critical and important as they encounter even mild childhood illnesses like a cold and can become very ill at those time, but also ongoing issues with their growth and development related to their cardiopulmonary condition and related to their birth defect otherwise in ways we don’t fully understand. Sometimes differences in their developmental trajectories also related to their cardiopulmonary condition and other aspects of the diaphragmatic hernia that we don’t understand. Then, sometimes, the need for additional surgeries or other genetic syndromes and genetic conditions that we are still learning more about every day.

We really see how a multidisciplinary clinic setting can really benefit those children where we bring together the surgeon, the neonatologist, the developmental specialist, the pulmonologist, the cardiologist or pulmonary hypertension team, nutritionists, and social workers, who can really comprehensively help us address all of those things that those families are facing and all the challenges those children might have and really working toward optimizing those children’s outcomes.

One example of this is that we did a randomized trial of fetuses with severe congenital diaphragmatic hernia many years ago and completed enrollment in that trial in 2001 with a follow-up planned out to two years of age. We completed that in 2003. Interestingly, what we learned from that trial was that even the children who we thought would do well based on their condition at discharge continued to have multiple medical problems and other challenges that were very, very important for those families and those children. There certainly were data out there to support that as well. We had the opportunity because we were funded in this trial to do the follow-up to really learn more about that and to recognize that we really needed to pay a lot more attention to our population of children with congenital diaphragmatic hernia as a whole, and so following that trial, we actually founded a multidisciplinary congenital diaphragmatic hernia clinic at UCSF at the Children’s Hospital with great support from our Department of Surgery and Pediatric Surgery Division.

We had started with a nurse coordinator, part time social worker, nutritionist. We worked together with a development specialist who did the high risk infant follow-up clinic for the intensive care nursery, and then myself and one of the surgeons. Through that ongoing experience, we’ve really built up the program to encompass even more services and more access for these families in terms of feeding therapy, which is a big part of the challenges that many of these children face, in addition to expanding the other services and bringing in the pulmonologist and the pulmonary hypertension service as well for those children who need additional help in those areas. That really has been a success story for children with this condition, which actually turns out to be much more complex, I think, than we really appreciated even going into our trial, which was in the late 1990s.

Another thing that I think we’ve seen is that as children who are born extremely pre-term are surviving with more severe forms of chronic lung disease complicated by pulmonary vascular disease and pulmonary hypertension, that many of these children are either taking quite a long time to get out of the hospital or they’re getting out of the hospital with really quite significant ongoing medical needs. The model for many centers has been to follow children in the developmental follow-up clinic, which may also have some additional support for their medical conditions or some institutions may have a bronchopulmonary dysplasia clinic where these children are seen, but oftentimes we, again, are lacking sort of the comprehensive multidisciplinary care that transitions through those long-term hospitalizations and into the outpatient setting.

I think that this has really been shown very nicely by the group at the University of Colorado to be an important contribution to the care of these patients providing this multidisciplinary care and transitioning through these long hospitalizations, sometimes moving from intensive care nursery to the pediatric ICU or another unit in the hospital, and then as an outpatient. That is a model that many of us are hoping to really emulate and to be able to provide the services these children need.

My name is Dr. Roberta Keller, and I’m aware that I’m rare.

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